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Chronic granulomatous disease: A multicenter study from the MENA region

Salem Al-Tamemi1, Najla Mekki2, Fatima Ailal3

  • 1Department of Child Health, Sultan Qaboos University Hospital, University Medical Center, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman.

Journal of Human Immunity
|July 8, 2026
PubMed

Insights

Chronic granulomatous disease (CGD) significantly impacts the MENA region, with common infections like pneumonia and aspergillosis. Early genetic testing and hematopoietic stem cell transplantation (HSCT) are crucial for better survival rates.

Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Chronic granulomatous disease (CGD) is a primary immunodeficiency affecting phagocyte function.
  • The Middle East and North Africa (MENA) region presents unique epidemiological characteristics for rare genetic disorders.
  • Understanding CGD's burden in the MENA region is vital for targeted healthcare strategies.

Purpose of the Study:

  • To comprehensively investigate the clinical spectrum, infectious agents, and outcomes of CGD patients in the MENA region.
  • To determine the genetic basis and inheritance patterns of CGD within this population.
  • To assess mortality and survival rates to inform clinical management and public health initiatives.

Main Methods:

  • A retrospective, multicenter study involving 322 patients diagnosed with CGD.
  • Data collection included clinical features, infection types, causative microorganisms, management strategies, and survival data.
  • Genetic analysis was performed to identify inheritance patterns (autosomal recessive vs. X-linked).

Main Results:

  • Pneumonia (58.0%), lymphadenitis (49.1%), and skin infections were most frequent. *Staphylococcus* and *Aspergillus* spp. were common pathogens.
  • Autosomal recessive inheritance was predominant (84.3%) over X-linked (15.7%).
  • Median survival was 8.5 years, with an estimated 10-year survival rate of 77.3%.

Conclusions:

  • CGD poses a substantial health challenge in the MENA region, characterized by specific infectious profiles and genetic predispositions.
  • Early diagnosis through molecular/genetic testing is essential for timely intervention.
  • Improving access to hematopoietic stem cell transplantation (HSCT) can significantly enhance patient survival and quality of life.

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